ALS2 Antibody (monoclonal) (M01)
Mouse monoclonal antibody raised against a partial recombinant ALS2.
- 产品详情
- 实验流程
- 背景知识
Application ![]()
| WB, E |
---|---|
Primary Accession | Q96Q42 |
Other Accession | BC029174 |
Reactivity | Human |
Host | mouse |
Clonality | monoclonal |
Isotype | IgG1 Kappa |
Clone Names | 4F9 |
Calculated MW | 183634 Da |
Gene ID | 57679 |
---|---|
Other Names | Alsin, Amyotrophic lateral sclerosis 2 chromosomal region candidate gene 6 protein, Amyotrophic lateral sclerosis 2 protein, ALS2, ALS2CR6, KIAA1563 |
Target/Specificity | ALS2 (AAH29174, 221 a.a. ~ 320 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. |
Dilution | WB~~1:500~1000 E~~N/A |
Format | Clear, colorless solution in phosphate buffered saline, pH 7.2 . |
Storage | Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing. |
Precautions | ALS2 Antibody (monoclonal) (M01) is for research use only and not for use in diagnostic or therapeutic procedures. |
For Research Use Only. Not For Use In Diagnostic Procedures.
Provided below are standard protocols that you may find useful for product applications.
BACKGROUND
The protein encoded by this gene contains an ATS1/RCC1-like domain, a RhoGEF domain, and a vacuolar protein sorting 9 (VPS9) domain, all of which are guanine-nucleotide exchange factors that activate members of the Ras superfamily of GTPases. The protein functions as a guanine nucleotide exchange factor for the small GTPase RAB5. The protein localizes with RAB5 on early endosomal compartments, and functions as a modulator for endosomal dynamics. Mutations in this gene result in several forms of juvenile lateral sclerosis and infantile-onset ascending spastic paralysis. Multiple transcript variants encoding different isoforms have been found for this gene.
REFERENCES
Personalized smoking cessation: interactions between nicotine dose, dependence and quit-success genotype score. Rose JE, et al. Mol Med, 2010 Jul-Aug. PMID 20379614.A novel ALS2 splice-site mutation in a Cypriot juvenile-onset primary lateral sclerosis family. Mintchev N, et al. Neurology, 2009 Jan 6. PMID 19122027.An interrupted beta-propeller and protein disorder: structural bioinformatics insights into the N-terminus of alsin. Soares DC, et al. J Mol Model, 2009 Feb. PMID 19023603.Maternal uniparental heterodisomy with partial isodisomy of a chromosome 2 carrying a splice acceptor site mutation (IVS9-2A>T) in ALS2 causes infantile-onset ascending spastic paralysis (IAHSP). Herzfeld T, et al. Neurogenetics, 2009 Feb. PMID 18810511.Novel homozygous ALS2 nonsense mutation (p.Gln715X) in sibs with infantile-onset ascending spastic paralysis: the first cases from northwestern Europe. Verschuuren-Bemelmans CC, et al. Eur J Hum Genet, 2008 Nov. PMID 18523452.

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